合并呼吸道梗阻的血管瘤和脉管畸形32例治疗分析

Treatment of hemangiomas and vascular malformations associated with airway obstruction: An assessment of 32 consecutive cases

目的:总结合并呼吸道梗阻的血管瘤和脉管畸形的临床特点和治疗经验。方法:1985年6月～2003年6月间,收治32例合并不同程度的呼吸道梗阻和呼吸困难的血管瘤和脉管畸形病例,男20例,女12例,年龄2个月～46岁,平均年龄9.31岁。其中血管瘤3例,静脉畸形12例,淋巴管畸形17例。病变原发于咽喉部4例,面颈部病变压迫或侵犯咽喉26例,上纵隔病变压迫气管2例。合并Ⅰ度、Ⅱ度、Ⅲ度呼吸困难分别为22例、7例、3例。采用口服糖皮质激素治疗3例,平阳霉素等硬化剂病变内注射治疗14例,手术治疗3例,手术+硬化剂病变内注射治疗12例。32例中,紧急气管切开4例,预防性气管切开11例。结果:3例血管瘤、16例淋巴管畸形和10例静脉畸形治愈;1例淋巴管畸形和2例静脉畸形好转;无效病例0例。结论:对合并呼吸道梗阻的血管瘤和压迫咽喉或气管的淋巴管畸形,分别采用激素疗法和单纯平阳霉素病变内注射治疗,治愈率高,一般不需气管切开;原发于咽喉部的局限性静脉畸形和淋巴管畸形手术容易治愈;侵犯咽喉部的静脉畸形和淋巴管畸形多合并面颈部广泛性病变,需采取手术+硬化剂注射+气管切开的综合治疗,方能治愈或控制病变发展。

PURPOSE: To summarize the clinical characteristics and treatment of hemangiomas and vascular malformations associated with airway obstruction. METHODS: From June 1985 to June 2003, 32 patients (3 cases with hemangioma, 12 cases with venous malformation and 17 cases with lymphatic malformation) associated with airway obstruction were treated. Among them, the primary lesions of 4 cases were in the laryngopharynx, 15 cases in the face and neck compressing the laryngopharynx, 11 cases in the face and neck with involvement of the laryngopharynx, 2 cases in the superior mediastinum compressing the trachea. 22 cases,7 cases and 3 cases had classⅠ,Ⅱand Ⅲ dyspnea respectively. 3 patients were treated with corticoids,14 patients received intralesional injection of Pingyangmycin,3 patients underwent operation,12 patients underwent operation and sclerosing therapy. Among 32 patients,4 had emergent tracheotomy and 11 had prophalactic tracheotomy. RESULTS: 3 cases with hemangioma, 16 cases with lymphatic malformation and 10 cases with venous malformation were cured,1 case with lymphatic malformation and 2 cases with venous malformation improved. CONCLUSION: For hemangioma and lymphatic malformation associated with airway obstruction that did not involve the laryngopharynx, conservative therapy such as oral corticoid or intralesional injection with Pingyangmycin alone could get an excellent result and most of the patients need no tracheotomy;for limited venous and lymphatic malformation of laryngopharynx,operation is a good choice;but for venous and lymphatic malformation that involved the laryngopharynx,usually associated with extensive lesion of head and neck,operation should be combined with sclerosing therapy and tracheotomy. [